颅颈区减压及后颅窝重建术治疗Chiari畸形合并脊髓空洞症

目的 探讨颅颈区减压及后颅窝扩大重建术治疗小脑扁桃体下疝畸形（ｃｈｉａｒｉ ｍａｌｆｏｍａｔｉｏｎ,ＣＭ）－脊髓空洞（ＳＭ）复合征的疗效。方法 对１９９４年至１９９９年收治的３７例ＣＭ－ＳＭ复合征患者,采取颅颈区减压、显微硬膜下探查松解、后颅窝扩大重建术,而不行空洞切开引流。结果 ３７例随访６个月至４．５年,症状改善３１例（８３．８％）;不变５例（１３．５％）,加重１例（２．７％）,无死亡;ＭＢＩ     

Arnold－Chiari畸形33例报告

本文报告３３例Ａｒｎｏｌｄ－Ｃｈｉａｒｉ畸形（ＡＣＭ），均经ＭＲＩ证实，其中合并脊髓空洞症２４例，颅颈交界处骨质畸形７例，脑积水６例．临床主要征象为枕大孔区压迫综合征。根据其临床表现、Ｘ线平片、ＣＴ和ＭＲＩ检查明确诊断。本组病人２例行保守治疗，２例行侧脑室－腹腔分流术，２９例行后颅窝开颅、上颈椎椎板切除术。结果好转２５例，无变化７例，恶化１例．本文就ＡＣＭ的发病机理、分型、诊断及治疗进行了简要讨论．     

Chiari-Ⅰ畸形的分类与治疗

１８９１年，Ｃｈｉａｒｉ首次描述ＣｈｉａｒｉⅠ畸形，其病理改变包括小脑扁桃体疝入枕大孔水平以下、脑干弯曲下移等。近年来随着影像学的不断进展，发现５０％～７０％的ＣｈｉａｒｉⅠ畸形病人合并脊髓空洞〔１〕。目前对本病的治疗无统一意见，方法杂乱繁多，如后颅...     

Chiari畸形伴脊髓空洞症的脑脊液动力学研究

在Ｃｈｉａｒｉ畸形中，除小脑病扁桃体直接压迫延，颈髓所致的病理生理改变外，一直为人关注的是ＣＳＦ的动力学变化在合并脊髓空洞症时的病理生理，本文报道７例Ｃｈｉａｒｉ畸形Ｉ型体脊髓空洞症患者，经ＭＲ证实，并由ＣＴ明确有无脑室扩大，全部病例行颈部的过伸，过屈，正常位置下的Ｑｕｅｃｋｅｎｓｔｅｄｔ试验（奎氏试验），并根据试验结果分别脑室一腹腔分流术，后颅窝Ｃ１椎板减压术和硬膜减张，术后随访上述测试，均有Ｃ     

Arnold—Chiari畸形33例报告

本文报告３３例Ａｒｎｏｌｄ－Ｃｈｉａｒｉ畸形，均经ＭＲＩ证实，其中合并脊髓空洞症２４例，颅颈交界处骨质畸形７例，脑积水６例。临床主要征象为枕大孔区压迫综合征，根据其临床表现，Ｘ线平片，ＣＴ和ＭＲＩ检查明确诊断。本组病人２例行保守治疗，２例行侧脑室－腹腔分流术，２９例行后颅窝开颅，上颈椎椎板切除术。     

脊髓

脊髓血管母细胞瘤的诊断和治疗；枕大池重建术治疗ChiariI型畸形合并脊髓空洞症；显微手术治疗小儿腰骶部脊髓脊膜膨出35例报告；颈椎前后路一期联合手术治疗颈髓损伤；枕大池重建术治疗ChiariI型畸形合并脊髓空洞症     

Arnold—Chiari畸形合并脊骨空洞症的显微外科治疗

目的：介绍Arnold-Chiari畸形合并脊髓空洞症（ACM-SM）显微外科治疗的术式及要点。方法：总结我院1997年1月-2000年12月25例ACM-SM显微外科治疗的经验。结果：25例ACM-SM术后临床症状改善23例（92%）。术后平均随访1.5年，获得随访19例，复查MRI小脑扁桃体下疝全部消失，均有人工枕大池形成，脊髓空洞消失13例，空洞明显缩小6例。结论：在颅后窝减压基础上，显微镜下松解小脑扁桃体与颈髓的粘连并切除，重建枕大池对治疗ACM-SM有明显疗效。     

枕颈融合联合扩大成形减压治疗枕颈区畸形

目的探讨枕颈融合联合扩大成形减压术在治疗枕颈区畸形中的应用价值。方法对18例枕颈区畸形患者行后路颈椎管扩大成形、脊髓减压、枕颈融合、取髂骨植骨内固定术。采用JOA评分评价患者功能改善情况。结果患者均获得随访,时间13～38个月。15例术后3个月植骨融合,3例术后6个月植骨融合。无脑脊液漏、脊髓损伤加重、脊髓空洞等并发症。术后12个月JOA评分为13～18(15. 42±0. 52)分,较术前8～12(11. 52±0. 79)分明显改善(P 0. 05),改善率为71%。结论枕颈融合联合椎管扩大成形术能有效解除枕颈段脊髓压迫、稳定上颈椎,是脊髓减压、重建上颈椎稳定性有效、可靠的方法。     

枕颈畸形和脊髓空洞症及其外科治疗

本文报告１３例颈枕部畸形合并脊髓空洞症。根据临床表现和影象学征象，设计并实施外科治疗。其外科基本操作过程：枕骨大孔扩大，寰椎后弓切除减压及枕骨和第二颈椎棘突基底部植骨融合术，脊髓空洞与蛛网膜下腔分流术，平均随访２５个月。其结果：症状和体征明显改善１０例，稍改善１例，无变化１例，因呼吸衰竭死亡１例。本文对枕颈畸形合并脊髓空洞症及其治疗方法作了讨论。     

显微硬脊膜减压治疗ChiariⅠ型伴脊髓空洞症

我院自 1997年～ 2 0 0 1年收治 2 5例Ｃｈｉａｒｉ畸形Ⅰ型伴脊髓空洞症(ｓｙｒｉｎｇｏｍｙｅｌｉａ ,ＳＭ)的患者 ,行颅颈区减压、显微镜下硬膜外层剥除、枕大池重建的手术治疗 ,疗效满意 ,现报告如下。1.临床资料与手术方法 :本组男 7例 ,女 18例 ;年龄 2 8～ 5 8岁 ;病程2个月～ 2 0 0年 ,平均 5 7年。所有患者术前均行头、颈、上胸段ＭＲＩ检查 ,证实为Ｃｈｉａｒｉ畸形Ⅰ型 ,小脑扁桃体下疝超过枕大孔后缘 6～ 15ｍｍ ,平均 12ｍｍ ;均合并有不同程度的ＳＭ ;2例伴轻至中度脑积水 ,1例伴脊柱侧弯。临床表现 :2 0例躯干及肢体麻木、…     

Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance

Thirty-five consecutive adults with Chiari malformation and progressive symptoms underwent surgical treatment at a single institution over a 3-year period. All patients underwent magnetic resonance imaging scan before and after surgery. Images of the craniovertebral junction confirmed tonsillar herniation in all cases and allowed the definition of two anatomically distinct categories of the Chiari malformation in this age group. Twenty of the 35 patients had concomitant syringomyelia and were classified as Type A. The remaining 15 patients had evidence of frank herniation of the brain stem below the foramen magnum without evidence of syringomyelia and were labeled Type B. Type A patients had a predominant central cord symptomatology; Type B patients exhibited signs and symptoms of brain stem or cerebellar compression. The principal surgical procedure consisted of decompression of the foramen magnum, opening of the fourth ventricular outlet, and plugging of the obex. Significant improvement in preoperative symptoms and signs was observed in 9 of the 20 patients (45%) with syringomyelia (Type A), as compared to 13 of the 15 patients (87%) without syringomyelia (Type B). Postoperative reduction in syrinx volume was observed in 11 of the 20 patients with syringomyelia, including all 9 patients with excellent results. Magnetic resonance imaging has allowed a classification of the adult Chiari malformation in adults based on objective anatomic criteria, with clinical and prognostic relevance. The presence of syringomyelia implies a less favorable response to surgical intervention.     

Transverse Microincisions of the Outer Layer of the Dura mater Combined with Foramen Magnum Decompression as Treatment for Syringomyelia with Chiari I Malformation

Summary Numerous surgical procedures have been proposed for treatment of syringomyelia associated with Chiari I malformation, but the optimal treatment has not yet been uniformly standardised. The main aim of the surgical treatment of syringomyelia/Chiari I complex is directed toward restoration of physiological cerebrospinal fluid dynamic at the craniovertebral junction. We report the surgical results of eight patients, affected by syringomyelia and Chiari I malformation, age range from 18 to 62 years, treated by bony foramen magnum decompression combined with transverse microincisions of the outer layer of the dura mater. In an average postoperative follow-up period of two years neurological symptoms and signs improved in seven patients. Postoperative Magnetic Resonance showed a decrease in size of the syrinx in seven patients. These results suggest that foramen magnum decompression combined with transverse microincisions of the outer layer of the dura 1) is an effective and safe treatment option for syringomyelia and Chiari I malformation, 2) corrects the circulatory disturbances of cerebrospinal fluid dynamic, 3) leads to a decrease in size of the syrinx and to a significant improvement in neurological signs and symptoms, 4) avoids complications of intradural approaches and syringosubarachnoid shunting.     

Long-term outcome of surgical management of adult Chiari I malformation

Chiari I malformation continues to inspire controversy. Debate still exists about surgical options. The aim of this study is to evaluate the long-term outcome of posterior fossa decompression procedure (PFD) in the treatment of adult Chiari I malformation, focusing on some factors or technical aspects which might influence the outcome. Forty-six adult patients with Chiari I malformation operated by PFD are the subject of this study. The group included 21 males and 25 females, with mean age of 37.4?years. Patients were divided into two groups: group I (32 cases) with syringomyelia and group II (14 cases) without syringomyelia. Group I was further subdivided into three subgroups according to the surgical procedure adopted: group Ia (12 cases) operated by PFD only, group Ib (14 cases) operated by PFD with fourth ventricular shunt, and group Ic (six cases) operated by PFD and syringosubarachnoid shunt. All cases included in group II were operated by PFD only. In group I, symptoms improved in 14 cases (43.8?%) and stabilized in 18 cases (56.3?%), whereas in group II, symptoms resolved in ten cases (71.4?%) and improved in four cases (28.6?%). Postoperative magnetic resonance imaging showed that the syrinx was resolved in 21 cases (65.6?%), improved in seven cases (21.9?%), and unchanged in four cases (12.5?%). Among the mean follow-up period (5.8?years), recurrence of symptoms occurred in five cases (10.9?%), all of them are included in group I, and were reoperated again. Posterior fossa decompression is recommended as the treatment of choice in adult Chiari I malformation with or without syringomyelia. The presence of syringomyelia predicts a less favorable response to surgical intervention. Syringosubarachnoid shunting did not improve the long-term outcome either clinically or radiologically. Implanting a fourth ventricular shunt in cases of syringomyelia associated with adhesions at the foramen of Magendie decreases the long-term incidence of recurrence significantly. For recurrent cases, re-exploration of the initial posterior fossa decompression is recommended before any consideration is given for direct management of the syrinx.     

Fulcrum-bending像与伴发脊髓空洞或(和)Chiari畸形脊柱侧凸的手术治疗

[目的]探讨Fulcrum-bending(支点弯曲位)像对伴发Chiari畸形和(或)脊髓空洞的脊柱侧凸手术治疗的帮助及新思路,并分析手术疗效。[方法]18例伴发脊髓空洞和(或)Chiari畸形的脊柱侧凸患者的治疗方案分两组:(1)牵引后前后路联合脊柱侧凸矫形手术(6例):对脊柱侧凸有手术矫形指征、伴发无明显神经损害的Chiari畸形和(或)脊髓空洞者,先行Halo颅骨牵引,1周后行脊柱侧凸矫形手术;(2)直接行后路脊柱侧凸矫形手术(12例):术前拍Fulcrum-bending像,并根据其结果对脊柱侧凸进行矫形。[结果]6例牵引后手术患者Cobb’s角平均矫正率为61%;6例柔软型侧凸患者直接后路手术患者均未出现神经并发症,Cobb’s角平均矫正率为60%,且术前Fulcrum-bending像与术后Cobb’s角相接近;6例僵硬型侧凸患者中1例出现轻微神经损害情况,后逐渐恢复,Cobb’s角平均矫正率为52%,手术存在过度矫正。[结论]对无明显神经损害的伴发脊髓空洞或ChiariI型畸形的脊柱侧凸柔软患者,不必行术前牵引而直接行后路手术,且可以获得与牵引后前后路联合手术类似的矫形效果。     

Syringo-subarachnoid shunt for syringomyelia associated with Chiari malformation (type 1)

Summary The authors report the surgical results of 28 patients with syringomyelia associated with Chiari malformation (type 1). 28 patients underwent 34 operative procedures. Syringo-subarachnoid shunt was performed in 28 patients, foramen magnum decompression with syringo-subarachnoid shunt in three, ventriculo-peritoneal shunt in one, terminal syringostomy in one, and foramen magnum decompression with terminal syringostomy in one. In an average postoperative follow-up period of 3 years and 9 months ranging from one year to 7 years and one month, neurological symptoms and signs improved in 24 out of 28 patients (82%). Some improvement was noted in sensory deficit and motor weakness. In 3 patients, the symptoms did not change. In 3 patients whose symptoms were unchanged, preoperative studies demonstrated atrophy of the spinal cord, in which irreversible changes were shown. The symptoms deteriorated in one patient. In one patient whose symptoms increased 3 months after syringo-subarachnoid shunt, shunt insufficiency due to postoperative adhesive arachnoiditis was responsible for neurological deterioration.The authors propose that syringo-subarachnoid shunt is effective as a surgical procedure for syringomyelia associated with Chiari malformation (type 1) if the patient does not have symptoms due to Chiari malformation or has only mild signs and symptoms which do not require foramen magnum decompression, such as nystagmus or atrophy of sternocleidomastoid muscle.     

Surgical treatment of syringomyelia. Selection of surgical procedures

The surgical approach to syringomyelia is controversial. In this study, the authors evaluated the results of various operative procedures applied in 31 patients with syringomyelia. Syringomyelia was associated with Chiari malformation in 17 cases, with spinal adhesive arachnoiditis in nine, with trauma in three, and with epidural arachnoid cyst in one. One case was idiopathic. A total of 38 operations were performed. Syringosubarachnoid shunting was applied in 17 patients, syringoperitoneal shunting in 11, terminal syringostomy in three, ventriculoperitoneal shunting in three, lumboperitoneal shunting in two, foramen magnum decompression (suboccipital craniectomy plus upper cervical laminectomy) and terminal syringostomy in one, and foramen magnum decompression with syringosubarachnoid shunting in one. The postoperative follow-up period ranged from 2 to 55 months (average, 26 months). Of the 31 patients, 23 showed neurological improvement, five were unchanged, and three deteriorated. Among the last three, one patient with Chiari malformation developed shunt malfunction due to arachnoiditis after syringosubarachnoid shunting. In two patients with syringomyelia secondary to adhesive arachnoiditis, the spinal cord was damaged by extensive separation of the arachnoid membrane at surgery. On the basis of the results in these 31 cases, the authors conclude that syringosubarachnoid shunting is effective for syringomyelia associated with Chiari malformation if syringomyelia is responsible for the clinical symptoms. Post-traumatic syringomyelia and syringomyelia secondary to adhesive arachnoiditis should be treated by syringoperitoneal shunting. If hydrocephalus is present, ventriculoperitoneal shunting is indicated. Finally, terminal syringostomy is no more effective than syringosubarachnoid or syringoperitoneal shunting.     

脊髓空洞症的手术治疗与临床评价

目的：对脊髓空洞症应用外科手术治疗进行探讨,以提高该种病变的临床疗效。方法：129例脊髓空洞症经CT或MRI扫描检查确诊,应用外科手术治疗,并进行平均1．5年以上的随访及评判治疗效果。结果：86例经受空洞－蛛网膜下腔分流术或空洞切开术的病人,空洞腔均显闭合,43例单纯行枕－颈减压的Amold-Chiari畸形或脊髓闭合不全的病人,其空洞腔亦见明显缩小。结论：在脊髓空洞症病人宜采取外科手术治疗,而枕－颈减压,空洞切开术或空洞－蛛网膜下腔分流术是可供选择有效治疗方法。     

Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION: A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE: Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION: We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.     

Circumferential decompression of the foramen magnum for the treatment of syringomyelia associated with basilar invagination

Posterior fossa decompression utilizing suboccipital craniectomy and duraplasty remains the standard surgical treatment for Chiari-associated syringomyelia. In the presence of basilar invagination, anterior decompression, typically transoral odontoidectomy, or posterior decompression may be performed. We report two cases in which anterior and posterior (circumferential) decompression of the foramen magnum was used to treat cervical syringomyelia successfully. These cases demonstrate that circumferential decompression of the foramen magnum may be necessary in some cases of cervical syringomyelia associated with basilar invagination and Chiari malformation.